Susac's syndrome: intratympanic therapy for hearing loss and a review of the literature

Laryngoscope. 2009 Jan;119(1):141-4. doi: 10.1002/lary.20040.

Abstract

Two cases of Susac's Syndrome are presented with a discussion of treatment modalities for the associated severe sensorineural deafness. Patients were managed with high dose oral steroids, anticoagulants and immunosuppressive drugs. The addition of intratympanic methylprednisolone injections were added to one patient's regimen with initial improvement but subsequent relapse and progression to profound deafness. The management of Susac's syndrome is difficult and still in need of innovative methods as the standard treatment continues to be ineffective in the long term.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Adult
  • Brain Diseases / complications
  • Brain Diseases / diagnosis
  • Diagnosis, Differential
  • Female
  • Glucocorticoids / administration & dosage
  • Glucocorticoids / therapeutic use*
  • Hearing Loss, Sensorineural / complications
  • Hearing Loss, Sensorineural / diagnosis*
  • Hearing Loss, Sensorineural / drug therapy*
  • Humans
  • Immunosuppressive Agents / administration & dosage
  • Immunosuppressive Agents / therapeutic use*
  • Retinal Artery Occlusion / complications
  • Retinal Artery Occlusion / diagnosis
  • Syndrome
  • Tympanic Membrane

Substances

  • Glucocorticoids
  • Immunosuppressive Agents